Cerebellar mutism syndrome and pilocytic astrocytoma

Case contributed by Antonio Rodrigues de Aguiar Neto
Diagnosis almost certain

Presentation

Quadriparesis, hypotonia, irritability, difficulty in swallowing, and absence of verbal response (mutism) after posterior fossa tumor resection surgery.

Patient Data

Age: 4 years
Gender: Female

Preoperative MRI shows large enhancing solid mass in the midline posterior fossa, centered in the fourth ventricle and involving the vermis, with resultant moderate obstructive supratentorial hydrocephalus. There is compression of the brainstem, with distortion of the dentate nucleus region and cerebellar peduncles, mostly on the right. Pathology was consistent with pilocytic astrocytoma.

Postoperative MRI reveals gross resection of the tumor and resulting decompression of the brainstem. However, hydrocephalus persists. There are postoperative bilateral T2-weighted signal abnormalities within the dentate nucleus and superior cerebellar peduncles, predominantly on the right.

Case Discussion

Cerebellar mutism syndrome may occur after resection of midline cerebellar or fourth ventricle tumors in the posterior fossa in the pediatric population 1,2,3. Postoperative MRI studies usually show bilateral T2-weighted signal abnormalities within the superior cerebellar peduncles and dentate nucleus 3. This case shows the clinical manifestations and radiological features of a postoperative cerebellar mutism syndrome in a pediatric patient. In this specific case, the patient's mutism symptoms improved significantly by 40 days.

Case courtesy

  • Sterfferson Morais, MD - PGY-3, radiology resident, Department of Radiology
  • Luiz Severo Bem Junior – PGY-4, neurosurgery resident, Department of Neurosurgery
  • Suzana M B Serra, MD – pediatric neurosurgeon, Department of Neurosurgery
  • Antonio Rodrigues de Aguiar Neto, MD - radiologist, Department of Radiology
  • Hospital da Restauração – Recife, PE – Brazil

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